Left isomerism with ipsilateral hepatic venous drainage to the right- and left-sided atria is rare and presents complex cardiac anatomy for surgical planning of biventricular repair. This case presents a biventricular repair, guided by 3D modeling, in a patient with left isomerism, balanced complete atrioventricular septal defect (CAVSD), interrupted inferior vena cava with azygos continuation, and ipsilateral hepatic venous drainage to the right- and left-sided atria. Postnatal echocardiogram and cardiac catheterization confirmed balanced CAVSD, normal filling pressures, pulmonary overcirculation, and elevated pulmonary vascular resistance, favoring staged repair with initial pulmonary artery banding. Patient-specific 3D models allowed direct observation of the complex venous drainage into the left-sided atrium and planning of an intra-atrial baffle. At 11 months, the patient underwent biventricular repair using a 2-patch technique and intracardiac baffling. Intraoperative imaging showed no residual shunt, excellent atrioventricular valve function, and unobstructed venous drainage. This case demonstrates the value of 3D modeling in guiding biventricular repair in complex anomalous venous anatomy.
Background The impact of prior sternotomies on pediatric heart transplant outcomes remains unclear. Given the increasing prevalence of reoperative candidates, clarifying this relationship is important for contemporary risk stratification. Methods We retrospectively analyzed 3253 pediatric heart transplant recipients in the Organ Procurement and Transplantation Network database. Pediatric heart transplant records from 1987 to 2025 were screened, and the analytic cohort was restricted to eligible primary transplants performed from 2002 to 2025. Patients were categorized as having no prior sternotomy (NS, n = 110), 1 to 2 prior sternotomies (1-2S, n = 2315), or ≥3 prior sternotomies (≥3S, n = 828), using clinically meaningful groupings supported by exploratory spline analysis. Results Recipients with ≥3 prior sternotomies were older, more often had congenital heart disease, and had higher body mass index, serum creatinine, and bilirubin, longer waitlist duration, and longer ischemic time. Pretransplant mechanical ventilation was less frequent in the ≥3S group. Posttransplant dialysis was more frequent in ≥3S than in 1 to 2S recipients, while treated acute rejection was more frequent in ≥3S than in both other groups. Stroke and permanent pacemaker implantation did not differ across groups. Survival at 30 days, 90 days, 1 year, and 5 years was lower in ≥3S than in 1 to 2S recipients; comparisons involving the smaller NS group were not statistically significant. In a prognostic multivariable logistic model incorporating recipient, transplant, and posttransplant factors, prior sternotomy burden was not independently associated with all-cause mortality at the most recent follow-up. Conclusion The number of prior sternotomies was associated with increased morbidity but was not independently associated with mortality in the prognostic multivariable model.
Objective Pectus excavatum is a congenital chest wall deformity characterized by anterior chest wall depression and can be observed in patients undergoing cardiac surgery. Historically, simultaneous repair of congenital heart defects and pectus excavatum has been avoided due to potential risks and complications. This study aimed to evaluate the safety and feasibility of simultaneous repair of congenital heart defects and pectus excavatum using the Nuss procedure. Methods This retrospective study reviewed 5 patients who underwent cardiac surgery concurrently with the Nuss procedure (Group A) and 142 patients who underwent only the Nuss procedure (Group B) between September 2000 and November 2021 at our institution. We compared the pre- and postoperative Haller index, operative time for the Nuss procedure, and the incidence of Nuss bar-related complications between Groups A and B. Results No complications related to the Nuss procedure, such as infection, bar displacement, cardiac injury, or recurrence of pectus excavatum, occurred in Group A. There were no statistically significant differences between the 2 groups in pre- and postoperative Haller index, Nuss operative time, or complication rates. No operative or late deaths occurred during the follow-up period in either group. Conclusions Simultaneous repair of congenital heart defects and pectus excavatum using the Nuss procedure appears to be feasible and does not seem to increase the risk of Nuss bar-related complications compared with isolated repair. While further studies are needed to evaluate the advantages of this strategy, this combined approach may be considered for carefully selected cardiac surgery candidates with severe pectus excavatum.
Introduction Reoperations following atrioventricular septal defect (AVSD) repair remain an important cause of late morbidity. This study aimed to evaluate long-term survival and identify risk factors associated with reoperation following AVSD repair. Methods A total of 395 patients underwent definitive AVSD repair between January 2010 and December 2021. The primary outcome was freedom from reoperation and identification of predictors of reoperation following index repair. Secondary outcomes included actuarial long-term survival and outcomes following reoperation. Results The median age at repair was 1.3(IQR 0.7-6.2) years. Complete AVSD repair accounted for 64.0% (253/395) of cases, while transitional and partial AVSD comprised 19.5% (77/395) and 16.5% (65/395), respectively. Freedom from reoperation was 95.8%, 92.9% and 86.8% at 1, 5 and 10 years, respectively. Twenty-eight of 395 patients(7.1%) underwent reoperation, including left atrioventricular valve (LAVV) repair in 14/28 (50.0%), LAVV replacement in 4/28 (14.3%), right atrioventricular valve (RAVV) repair in 6/28 (21.4%) and left ventricular outflow tract obstruction (LVOTO) resection in 5/28 (17.9%). Independent predictors of LAVV reoperation were age <3 months at initial repair (OR 4.03, 95% CI: 1.55-10.51, P = .004), double-orifice LAVV repair (OR 6.85, 95% CI: 2.59-18.12, P < .001) and residual moderate or greater LAVV regurgitation at discharge (OR 8.20, 95% CI: 3.59-21.76, P < .001). No significant predictors were identified for RAVV or LVOTO reoperation. Actuarial survival at 1, 5 and 10 years was 98.6%, 97.2% and 95.2%, respectively. No early or late mortality occurred among patients undergoing reoperation. Conclusion AVSD repair provides excellent long-term survival and low reoperation rates. Residual moderate LAVV regurgitation at discharge is the strongest predictor of reoperation. Durable cleft repair remains critical for long-term valve function. Most patients requiring LAVV reoperation are amenable to re-repair.
Objective(s) Patients with Turner syndrome (TS) and hypoplastic left heart syndrome (HLHS) undergoing single-ventricle palliation represent a challenging cohort. We examined birth admission outcomes in TS/HLHS patients, comparing Norwood and hybrid palliation strategies. Methods The pediatric health information system (PHIS) database was queried for neonates with a diagnosis of TS and HLHS. Cohort was divided into Norwood procedure, hybrid, or nonoperative, and outcomes were divided into three time eras (Era 1: 2004-2011, Era 2: 2012-2018, Era 3: 2019-2025). Results A total of 157 infants were identified: 27/157 (17%) received no operative intervention, 24/157 (15%) underwent hybrid palliation, and 106/157 (68%) underwent the Norwood procedure only. Overall Stage-1 palliation (S1P) hospital survival was 75% (98/130) for both operative cohorts. Hybrid palliations trended upward over time, while Norwood volumes remained stable. In-hospital survival improved across eras for hybrid palliation (75% vs 87%) and the Norwood procedure (71% vs 89%), although neither reached statistical significance. Of 45 PHIS hospitals performing the Norwood, 38 performed S1P for TS patients; 60% (23/38)used the Norwood exclusively, 8% (3/38) used hybrid exclusively, and 32% (12/38) employed both approaches. Conclusions Outcomes appear satisfactory with either the hybrid or Norwood strategy, with current survival rates ≥87%, although hybrid palliation was associated with a higher rate of subsequent transplantation. However, 17% of (27/157) TS/HLHS neonates were not offered surgical intervention, presumably because they were deemed to be at higher risk. Given demonstrably improving outcomes among selected TS patients undergoing either procedure, offering S1P more broadly, including to higher-risk TS/HLHS patients, may be worthwhile.
